REVIEW Tourette Syndrome Deep Brain Stimulation: A Review and Updated Recommendations Lauren E. Schrock, MD,1 Jonathan W. Mink, MD, PhD,2 Douglas W. Woods, PhD,3 Mauro Porta, MD,4 Dominico Servello, MD,5 Veerle Visser-Vandewalle, MD, PhD,6 Peter A. Silburn, MD,7 Thomas Foltynie, MRCP, PhD,8 Harrison C. Walker, MD,9 Joohi Shahed-Jimenez, MD,10 Rodolfo Savica, MD,1 Bryan T. Klassen, MD,11 Andre G. Machado, MD,12 Kelly D. Foote, MD,13 Jian-Guo Zhang, MD, PhD,14 Wei Hu, MD, PhD,11,14 Linda Ackermans, MD, PhD,15 Yasin Temel, MD, PhD,15 Zoltan Mari, MD,16 Barbara K. Changizi, MD,17 Andres Lozano, MD,18 M. Auyeung, MD,19 Takanobu Kaido, MD, PhD,20 Yves Agid, MD, PhD,21 Marie L. Welter, MD, PhD,22 Suketu M. Khandhar, MD,23 Alon G. Mogilner, MD, PhD,24 Michael H. Pourfar, MD,24 Benjamin L. Walter, MD,25 Jorge L. Juncos, MD,26 Robert E. Gross, MD,27 Jens Kuhn, MD,28 James F. Leckman, MD,29 Joseph A Neimat, MD,30 Michael S. Okun, MD,13* on behalf of the and Tourette Syndrome Association International Deep Brain Stimulation (DBS) Database and Registry Study Group 1Department of Neurology, University of Utah, Salt Lake City, Utah, USA 2Department of Neurology, University of Rochester Medical Center, Rochester, New York, USA 3Department of Psychology, Texas A&M University, College Station, Texas, USA 4Tourette Centre, IRCCS Galeazzi Hospital, Milan, Italy 5Functional Neurosurgical Unit, IRCCS Galeazzi Milano, Milan, Italy 6Department of Stereotactic and Functional Neurosurgery, University of Cologne, Cologne, Germany 7Royal Brisbane and Women’s Hospital, School of Medicine, University of Queensland, Brisbane, Queensland, Australia 8University College London Institute of Neurology, London, United Kingdom 9Department of Neurology, University of Alabama Birmingham, Birmingham, Alabama, USA 10Department of Neurology, Baylor College of Medicine, Houston, Texas, USA 11Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA 12Center for Neurological Restoration, Neurological Institute, Cleveland Clinic, Cleveland, OH, USA 13Departments of Neurology, Neurosurgery, and Psychiatry, University of Florida Center for Movement Disorders and Neurorestoration, Gainesville, Florida, USA 14Department of Neurosurgery, Beijing Tiantan Hospital, Capital University of Medical Sciences, Beijing, China 15Department of Neurosurgery, Maastricht University Medical Center, The Netherlands 16Department of Neurology, Johns Hopkins University, Baltimore, Maryland, USA 17Departments of Neurology and Psychiatry, The Ohio State University Wexner Medical Center, Ohio, USA 18Division of Neurosurgery, University of Toronto, Toronto, Ontario, Canada 19Department of Medicine, Pamela Youde Nethersole Eastern Hospital, Hong Kong, SAR China 20Department of Neurosurgery, National Center Hospital, National Center of Neurology and Psychiatry (NCNP), Tokyo, Japan 21Institut du Cerveau et de la Moelle Epinie` re (ICM), CHU Pitie-Salp etrie^ ` re, Paris, France 22Centre de Recherche de l’Institut du Cerveau et de la Moelle epinie ` re (CRICM), Universite Pierre et Marie Curie-Paris 6, Paris, France 23Northern California Kaiser Permanente, Surgical Movement Disorders Program, Sacramento, California, USA 24Departments of Neurosurgery and Neurology, New York University, Langone Medical Center, New York, New York, United States of America 25Movement Disorders Center, Neurological Institute, University Hospitals and Case Western Reserve University School of Medicine, South Euclid, Ohio, USA 26Department of Neurology, Emory University School of Medicine, Atlanta, Georgia, USA 27Department of Neurosurgery, Emory University School of Medicine, Atlanta, Georgia, USA 28Department of Psychiatry and Psychotherapy, University of Cologne, Cologne, Germany 29Child Study Center and the Yale Center for Clinical Investigation, Yale University School of Medicine, New Haven, Connecticut, USA 30Department of Neurological Surgery, Vanderbilt University Medical Center, Nashville, Tennessee, USA ------------------------------------------------------------------------------------------------------------------------------ *Correspondence to: Michael S. Okun, MD, 3450 Hull Road, Department of Neurology, Center for Movement Disorders and Neurorestoration, Gaines- ville, FL 32607, E-mail: [email protected]fl.edu Funding agencies: Relevant conflicts of interest/financial disclosures: Nothing to report. Author roles may be found in the online version of this article. Received: 4 April 2014; Revised: 6 October 2014; Accepted: 8 October 2014 Published online 00 Month 2014 in Wiley Online Library (wileyonlinelibrary.com). DOI: 10.1002/mds.26094 Movement Disorders, Vol. 00, No. 00, 2014 1 SCHROCK ET AL ABSTRACT: Deep brain stimulation (DBS) may nized and addressed. We have removed the previously improve disabling tics in severely affected medication suggested 25-year-old age limit, with the specification and behaviorally resistant Tourette syndrome (TS). Here that a multidisciplinary team approach for screening is we review all reported cases of TS DBS and provide employed. A local ethics committee or institutional updated recommendations for selection, assessment, review board should be consulted for consideration of and management of potential TS DBS cases based on cases involving persons younger than 18 years of age, the literature and implantation experience. Candidates as well as in cases with urgent indications. Tourette should have a Diagnostic and Statistical Manual of Men- syndrome patients represent a unique and complex tal Disorders, Fifth Edition (DSM V) diagnosis of TS with population, and studies reveal a higher risk for post- severe motor and vocal tics, which despite exhaustive DBS complications. Successes and failures have been medical and behavioral treatment trials result in signifi- reported for multiple brain targets; however, the optimal cant impairment. Deep brain stimulation should be surgical approach remains unknown. Tourette syndrome offered to patients only by experienced DBS centers DBS, though still evolving, is a promising approach for after evaluation by a multidisciplinary team. Rigorous a subset of medication refractory and severely affected preoperative and postoperative outcome measures of patients. VC 2014 International Parkinson and Movement tics and associated comorbidities should be used. Tics Disorder Society and comorbid neuropsychiatric conditions should be optimally treated per current expert standards, and tics Key Words: Tourette syndrome; DBS; guidelines; should be the major cause of disability. Psychogenic deep brain stimulation tics, embellishment, and malingering should be recog- Tourette syndrome (TS) is a chronic neurodevelop- with TS DBS provided suggested updates (Table 2) mental disorder characterized by motor and phonic and a consensus opinion on the TSA recommendations tics that by definition occur with a childhood onset.1 put in place in 2006.7 The syndrome is commonly associated with other neu- ropsychiatric comorbidities (eg, attention deficit hyper- activity disorder [ADHD], obsessive compulsive Literature Review features [OCD], and other behavioral manifestations). Deep brain stimulation is an established treatment In most TS cases, the motor manifestations can be for Parkinson’s disease (PD),8-12 essential tremor,13,14 managed using TS education, comprehensive behav- dystonia,15-17 and obsessive-compulsive disorder,18,19 ioral intervention for tics (CBIT), or a variety of medi- and has been granted either full Food and Drug Admin- cations.2-4 The natural history of TS is that most istration (FDA) approval (PD, essential tremor), or a patients will experience improvement of tics in late humanitarian device exemption (FDA; dystonia, OCD) adolescence or early adulthood.5,6 However, a subset for each of these indications (in the United States). of patients will continue to experience disabling tics Many studies and position papers detail careful and despite optimal medication and behavioral manage- meticulous techniques for screening patients for ment. For severely affected patients, deep brain stimu- DBS,13,16,20-22 including a few reports for TS lation (DBS) has the potential to improve refractory patients.23,24 General guidelines for selecting individual and disabling symptoms. TS patients for DBS therapy and for managing them preoperatively and postoperatively will have the poten- Methods tial to improve risk–benefit ratios. The importance of rigorous preoperative assessment, patient selection, DBS An experienced group of physicians participating in team expertise and experience, as well as postoperative the Tourette Syndrome Association (TSA) Interna- management has been demonstrated previously, espe- tional DBS Database/Registry, and also actively cially when groups have studied cohorts of patients involved in managing TS DBS patients, reviewed the who have failed DBS therapy.25,26 Because TS is a 2006 TSA guidelines7 and examined all reported cases childhood-onset disorder, often with complex clinical of TS DBS. The TSA Database/Registry group features, a waxing and waning course, and frequent accepted complete information sets on outcomes from neuropsychiatric comorbidities, the evaluation of TS DBS (database entry), as well as registration of patients has a greater level of complexity than many of cases performed with or without outcome information the other current DBS indications. (registry entry). The group summarized the literature In 2005, the TSA hosted a meeting of physicians (Table 1) and based on their collective experience with experience